Myelomeningocele, a severe form of spina bifida, occurs in approximately 1 in 3,000 live births in the United States. The extent of disability is generally related to the level of the myelomeningocele defect, with a higher upper level of lesion generally corresponding to greater deficits. Open maternal-fetal surgery for myelomeningocele repair is a major procedure for the woman and her affected fetus. Although there is demonstrated potential for fetal and pediatric benefit, there are significant maternal implications and complications that may occur acutely, postoperatively, for the duration of the pregnancy, and in subsequent pregnancies. Women with pregnancies complicated by fetal myelomeningocele who meet established criteria for in utero repair should be counseled in a nondirective fashion regarding all management options, including the possibility of open maternal-fetal surgery. Maternal-fetal surgery for myelomeningocele repair should be offered only to carefully selected patients at facilities with an appropriate level of personnel and resources.